4.8 Article

Alpha1-Antitrypsin Deficiency

期刊

NEW ENGLAND JOURNAL OF MEDICINE
卷 360, 期 26, 页码 2749-2757

出版社

MASSACHUSETTS MEDICAL SOC
DOI: 10.1056/NEJMcp0900449

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资金

  1. GlaxoSmithKline
  2. AstraZeneca and serving on advisory boards for the Alpha-1 Foundation
  3. Kamada
  4. Biotherapeutics
  5. CSL Behring

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A 60-year-old white man presents for evaluation of progressive dyspnea. He is a former smoker with a 20-pack-year smoking history and a 10-year history of diagnosed chronic obstructive pulmonary disease (COPD). There is no family history of COPD. Severe airflow obstruction is seen on spirometry, with a forced expiratory volume in 1 second (FEV1) that is 40% of the predicted value. Should the patient be evaluated for alpha(1)-antitrypsin (AAT) deficiency? If AAT deficiency is documented, how should his case be managed?

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